82,065 research outputs found

    Evaluating quality of life tools in North American patients with erythropoietic protoporphyria and X-linked protoporphyria.

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    BackgroundErythropoietic protoporphyria (EPP) and X-linked Protoporphyria (XLP) are rare photodermatoses presenting with severe phototoxicity. Although anecdotally, providers who treat EPP patients acknowledge their life-altering effects, tools that fully capture their impact on quality of life (QoL) are lacking.MethodsAdult patients with EPP/XLP were given four validated QoL tools: the Patient Reported Outcomes Measurement Information System 57 (PROMIS-57), the Hospital Anxiety and Depression Scale (HADS), the Illness Perception Questionnaire Revised (IPQR), and an EPP-Specific tool. All patients received the PROMIS-57 while the HADS, IPQR, and EPP-Specific tools were introduced at a later date. Associations between responses and clinical phenotypes were explored.ResultsTwo hundred and two patients were included; 193 completed PROMIS-57, 104 completed IPQR, 103 completed HADS, and 107 completed the EPP-Specific tool. The IPQR showed that patients strongly believed EPP/XLP had a negative impact on their lives. Mean scores in anxiety and depression domains of both HADS and PROMIS-57 were normal; however, anxiety scores from HADS were borderline/abnormal in 20% of patients. The EPP-Specific tool revealed a decreased QoL in most patients. The PROMIS-57 showed that 21.8% of patients have clinically significant pain interference. Several tool domains correlated with measures of disease severity, most being from the PROMIS-57.ConclusionsImpaired QoL is an important consequence of EPP/XLP. PROMIS-57 was most sensitive in evaluating impaired QoL in EPP/XLP. Further research is needed to compare the effectiveness of it for assessing response to treatment

    UK experience of liver transplantation for erythropoietic protoporphyria

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    Erythropoietic protoporphyria (EPP) is characterised by excess production of free protoporphyrin from the bone marrow, most commonly due to deficiency of the enzyme ferrochelatase. Excess protoporphyrin gives rise to the cutaneous photosensitivity characteristic of the disease, and in a minority of patients leads to end-stage liver disease necessitating liver transplantation (LT). There is limited information regarding the timing, impact and long-term outcome of LT in such patients, thus we aimed to identify the indications and outcomes of all transplants performed for EPP in the UK using data from the UK Transplant Registry. Between 1987 and 2009, five patients underwent LT for EPP liver disease. Median follow-up was 60 months, and there were two deaths at 44 and 95 months from causes unrelated to liver disease. The remaining recipients are alive at 22.4 years, 61 months and 55 months after transplant. A high rate of postoperative biliary stricturing requiring multiple biliary interventions was observed. Recurrent EPP-liver disease occurred in 4/5 (80%) of patients but graft failure has not been observed. Given the role of biliary obstruction in inducing EPP-mediated liver damage, we suggest that consideration should be given for construction of a Roux loop at the time of transplant. Thus we demonstrate that although EPP liver transplant recipients have a good long-term survival, comparable to patients undergoing LT for other indications, biliary complications and disease recurrence are almost universal, and bone marrow transplantation should be considered where possible

    Employer Pension Plan Inequality in Canada

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    The purpose of this research paper is to contribute to knowledge regarding employer pension plan (EPP) inequality in Canada. Information on EPP coverage and value is analyzed using the 1999 and 2005 Surveys of Financial Security. The results indicate that women, persons who may live alone, landed immigrants, and language minorities are at a disadvantage in their EPP coverage and accrued value. In addition, age, educational attainment, occupation, industry of employment, union membership, total personal income, province, and size of urban residence figure importantly in EPP coverage. Furthermore, age, educational attainment, industry of employment, total personal income, province and size of urban residence are all-important determinants of the termination value of EPPs. To identify inequalities in EPP coverage among the sub-populations, the researchers use multivariate analysis. This allows an identification of inequalities that are not a direct result of differences in age, gender, level of education, location, or position in the labour market. Findings indicate that differences in EPP coverage for women, persons who may live alone, landed immigrants and language minorities are primarily due to differences in these other characteristics. However, the lower EPP value witnessed by these subpopulations cannot be explained by individual or labour market characteristics.Employer Pension Plans, Registered Pension Plans, coverage, value, inequality, seniors, landed immigrants, gender differences, minority language

    Employer Pension Plan Inequality in Canada

    Get PDF
    The purpose of this research paper is to contribute to knowledge regarding employer pension plan (EPP) inequality in Canada. Information on EPP coverage and value is analyzed using the 1999 and 2005 Surveys of Financial Security. The results indicate that women, persons who may live alone, landed immigrants, and language minorities are at a disadvantage in their EPP coverage and accrued value. In addition, age, educational attainment, occupation, industry of employment, union membership, total personal income, province, and size of urban residence figure importantly in EPP coverage. Furthermore, age, educational attainment, industry of employment, total personal income, province and size of urban residence are all important determinants of the termination value of EPPs. To identify inequalities in EPP coverage among the sub-populations, the researchers use multivariate analysis. This allows an identification of inequalities that are not a direct result of differences in age, gender, level of education, location, or position in the labour market. Findings indicate that differences in EPP coverage for women, persons who may live alone, landed immigrants and language minorities are primarily due to differences in these other characteristics. However, the lower EPP value witnessed by these sub-populations cannot be explained by individual or labour market characteristics.Employer Pension Plans, Registered Pension Plans, coverage, value, inequality, seniors, landed immigrants, gender differences, minority language
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